대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

CLIPPERS (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Response to Steroids)

Radiologic Findings

Fig 1. Chest PA shows multiple tiny nodular opacities in both upper lung zones.
Fig 2-5. CT scans reveals multifocal irregular ill-defined nodules in both lungs. Some of the nodules are subsolid and some are solid.
Fig 6-7. Brain MRI and Spine MRI show multifocal nodular enhancing lesions at pons and spinal cord.the

Brief Review

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a CNS inflammatory disorder characterized by pontine predominant, punctate, gadolinium enhancing MRI lesions with exquisite response to corticosteroid therapy. The clinical manifestation includes brainstem symptoms, gait ataxia, and diplopia with subacute presentation.
Diagnosis requires careful exclusion of alternative diagnoses. The characteristic MRI pattern is pontine and cerebellar punctate gadolinium enhancement. Some reports show extrapontine lesions such as spinal cord, generalized lymphadenopathy, or lung lesions. On 1 case report, multiple solid pulmonary nodules were noted, showing central GGO surrounded consolidation, characterizing “reversed halo” pattern. Numerous and confluent nodules were in predominantly peribronchovascular distribution. The differential diagnosis includes vasculitis, sarcoidosis and angiocentric lymphoma. Due to lack of diagnostic criteria, clinical, radiological and pathological correlation is needed. On pathology, perivascular and T cell-predominant inflammatory cell infiltrates in affected lesion. Corticosteroids is recommended treatment, and response is key to diagnosis.

References

1. W Oliver Tobin, Yong Guo, Karl N Krecke, Joseph E Parisi, Claudia F Lucchinetti, Sean J Pittock, Jay Mandrekar, Divyanshu Dubey, Jan Debruyne, B Mark Keegan, Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), Brain, Volume 140, Issue 9, September 2017, Pages 2415–2425
2. Osborn et al., Diagnostic Image: Brain, 3rd edition, 770-771.
3. Mariana S L Pessoa, Francisca E M M de Lima, Luiz Ricardo A Uchôa, Danielle M Torres, Pablo Picasso de A Coimbra, Daniel G F Távora, Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) With Extrapontine and Pulmonary Involvement: Case Report of a Rare Presentation and Review of Literature, Neurol. India 2022; 70(1):405–408

Keywords